COPI is a key mediator of protein trafficking within the secretory pathway. COPI is recruited to the membrane primarily through binding to Arf GTPases, upon which it undergoes assembly to form coated transport intermediates responsible for trafficking numerous proteins, including Golgi-resident enzymes. Here, we identify GORAB, the protein mutated in the skin and bone disorder gerodermia osteodysplastica, as a component of the COPI machinery. GORAB forms stable domains at the trans-Golgi that, via interactions with the COPI-binding protein Scyl1, promote COPI recruitment to these domains.
Pathogenic GORAB mutations perturb Scyl1 binding or GORAB assembly into domains, indicating the importance of these interactions. Loss of GORAB causes impairment of COPI-mediated retrieval of trans-Golgi enzymes, resulting in a deficit in glycosylation of secretory cargo proteins.
Our results therefore identify GORAB as a COPI scaffolding factor, and support the view that defective protein glycosylation is a major disease mechanism in gerodermia osteodysplastica. COPI (coat protein complex I)-coated transport vesicles mediate protein trafficking in the early secretory pathway. They are responsible for retrograde transport from the Golgi apparatus to the endoplasmic reticulum (ER), and for trafficking between cisternae within the Golgi apparatus. Within the Golgi, COPI-coated vesicles mediate retrograde traffic of Golgi resident enzymes, and may also participate in anterograde trafficking of certain cargoes. Although COPI is best known for its role in vesicle trafficking, recent studies also suggest possible involvement in trafficking via tubular intermediates at the level of the Golgi stack. In line with its trafficking functions, COPI is localized at the ER-to-Golgi intermediate compartment (ERGIC) and Golgi apparatus, where it is abundant at the cisternal rims and enriched towards the cis-side.
The COPI coat is comprised of the hetero-heptameric coatomer complex, which is recruited from the cytosol to the membrane by the small GTPase Arf1, which itself is recruited from the cytosol concomitant with guanosine 5'-triphosphate (GTP) loading. Coatomer functions to both select cargo and promote vesicle formation, which is facilitated by the assembly of coatomer complexes into a cage-like structure.
As someone who made many pictures with fixed prime lens analogue cameras in the past (Rolleiflex - still my fave camera ever, 3 medium format fuji rangefinders, a nikon 35), I can definitely see the appeal.For me 35mm would have been ideal as the base length, allowing for more resolution when cropping. But I could live with its 28mm.For my general walkaround use I see only one useful feature missing: gps. It is so pleasant when photos are automatically geotagged, that is a great plus of mobile photos. Have an original RX-1 with EVF and it is still very satisfying.If I was in the market, Q2 would be a strong contender.
But I prefer 35mm lens and have doubts that a cropped 28mm is actually as good. Photonics and information theory says it is not, in part because the aperture is less in the 28mm (per f-stop and angular area). But advances in sensors probably make up for some of the loss; e.g. Base ISO = 50 implies a deeper full well, which compensates for less aperture via longer exp. (Why do I think that another raging 'equivalence' debate may be triggered? )Then there is the angular resolution differences between the 28mm vs 35mm lenses. Would need some rigorous tests to resolve that issue.
Anders:'replace'?No, arjun is setting up a good number of false equivalences. This lens always has the DoF characteristics of a 28mm f/1.7 full framed lens. And the DoF changes for any lens depending on how far/near the film plane is to the subject.' For a 75mm on a Leica M you need to stop down to f4.6 to obtain the same DOF as with the 28mm on the Q2.' This may be true, but it only works if the framing stays consistent.
Again besides f-stop and sensor area, DoF is highly dependent, not on the framing, but on how far the film plane is from the subject.I suspect what arjun is trying to say is that with the distance from the subject to the film plane UNCHANGED and the lens wide open a crop to the 75mm field of view will have the DoF of a 75mm lens shot at f/4.6.So it's only a very illustrative point if the distance from subject to film plane (sensor) factor is included. I don't think they are false equivalents at all. Depth of field on a 28mm lens is always going to be relatively deep - crop it, and that 28/1.7 dof doesn't change. Calculate back from that depth to the various crops and that's where he gets his equivalents fromYou are exactly right when you say: 'I suspect what arjun is trying to say is that with the distance from the subject to the film plane UNCHANGED and the lens wide open a crop to the 75mm field of view will have the DoF of a 75mm lens shot at f/4.6.'
But think about it, even as you move closer, it will still have that equivalent depth of field when cropped to 75mm, however, obviously, even a 75/4.6 lens will give a narrowish depth of field when at macro distances. Snoddas:'If you have been starving for a month I bet you'd prefer a good meal before a Q2'Nothing to do with anything I said.Besides food, somethings one needs before a car or a camera/lens, potable water, often shelter, usually some form of clothes.So massive false equivalence, whereas all I did was point out cars cost real money to run and maintain, even if you own them and digital cameras cost little once you own them.Now, say you need a car, well a Toyota or Ford is just fine. Say you need a camera+lens, well a Canon or Nikon is just fine. But say you want a really high performance car just for your ego, no need, well then that's several hundred thousand dollars to about two million.Want, or need for professional reasons, exotic camera lens performance for your photography, then that's a few thousand dollars to an upper limit of about $50,000 for one lens and one body. Notice how the Leica Q2 is near the bottom of that range. Snoddas:It's ridiculous that you think there's a street Ferrari which is analogous to very highend Leica optics; there isn't.
The best street Ferraris have real competition; the very best Leica lenses don't.It's ridiculous that you supposed I wasn't limiting my comment to the Q's lens. I never pointed to the D5 and said 'the Q competes with that.' You obviously don't know the difference between an ILC body and the Q, still.Now, since the Q's lens is just an excellent, not extraordinary, Leica lens, the very highend sports car analogy is perfect, since there is optical competition for this Q lens.The more you post the more your ignorance of cameras and lenses is clear.Next time you don't understand something (like what an ILC is) you can ask.Now about that Nikon Coolpix A. I have traveled a lot of places and have hardly ever seen a Leica. Even in Berlin which I have visited many times, I don't recall having ever seen a Leica.The last 15 years or so, I have maybe seen a Leica 3 to 4 times and I actually look out for the cameras people use, which is a good indication compared to all the 'highly popular' statements you read here and there.The vast majority of 'real' cameras I see are from Canon and Nikon, which include everything from low to high end cameras, and the rest are a few other brands like Fuji and Olympus/Panasonic 4/3 cameras.
AKH,I've seen Leicas on the street for the last 5 years.Last week, Monday I think, I saw someone with an SL with an M lens mounted on it. Yes, the M lens was a Leica M. Does that count as a double sighting?Technically this occured in the public area of a large retailer, so that wasn't on the street.
Oh, and the guy was over 40 and not of east Asian origin. So totally un-hipster.Yes, I've seen women carrying Ms too.Some times I see film Ms in use. As in people clicking the shutter on the street.In contrast, in say the last 15 months, I've seen perhaps 2 Hasselblad X1Ds and 1 Fuji GFX-50s out on the street.Once about a year ago, two guys (neither of Asian origin) separately had Ms at the big table we were sharing in one of the major public libraries in my city. One took some photos with his M.
I do see them knocking about but you probably won’t see as many of them as you might a Sony, Panasonic, Canon, Nikon as they are mass produced products. The Q and Leica cameras in general are not.
My Q is great, takes super photos and I take it everywhere.I’ve got Canon kit too and it’s better for certain things and has served me well. It’s not a Leica though. Highly recommend piece of kit which doesn’t owe me a penny as it’s been well used for 4 years and hopefully plenty more. Fancy the new version though!
some of The Fuji bodies offer comparable ergonomicsClose, but no cigar. Try manual focusing on an X100F. image quality is largely about what you like and what glass you’re using.Again, using the X100F for comparison, since it's the closest match, Fuji's cropped sensor and only above average optics can't hold a candle to the Leica.
Fuji's AF, though decent, is nowhere near as fast or accurate as the Q's. And let's not even mention the vast differences in materials, build quality, and ruggedness between the two.It is far, and accurate, to state that Leica cameras are 'overpriced' to some degree. But the fact remains that there is nothing one the market to compare with he Q or Q2, at any price. I'm not talking about just image quality but the overall design and quality of the product. FuzzyDice:'I dunno, I think some of The Fuji bodies offer comparable ergonomics, image quality is largely about what you like and what glass you’re using.' Image quality is largely a lens thing. And Fuji really doesn't have lenses equal to this Q's lens.The Fuji X3 ergonomics have nothing on this Q.'
Also, Leica Q market share has nothing to do with my feelings, last time I checked they weren’t top 3 in sales.' Is that supposed to be a joke? Make up something I didn't say, and then pretend your 'data' refutes me?You've lied about my point. Nowhere did I pretend that the Q has outsold for example a Rebel whatever from Canon.I didn't say 'the Q is a top selling camera', I said 'it's sold really well for Leica'. I'd bet it's sold better than all 3 versions of the Sony RX1 combined.Why is it Leica bashers like you are so ignorant of Leica gear, and separately have to tell flagrant lies to 'affirm' your assertions?
I really don't know the answer to these questions. My friend said that the 28/1.7 on Leica Q actually is the 24/1.7 but Leica cropped the area of the lens to avoid blurry edge of the images that the lense produces. Is this true?Apart from that thing, I have tried the Q2 this morning at the shop. After got home, checked the files, I have to say that the image quality dissappointed me quite a bit.
However, I did not say that the Q2 is bad. I love the controls, the feeling of turning the rings and how it operates. It is amazing, I almost could not resist to buy it. Just only the image quality that makes me hesitated. Apestorm:I don't live in the UK. And I've not been there in more than 20 years.Also stores usually sell stock-unless Leica (or say Nikon and the D850) has extras to give out to stores for demo.So no, it's not entirely believable that the Q2 is on display in Hong Kong, the UK, or the middle of Europe.The main Leica store in my city doesn't have one. But the store isn't actually owned by Leica.
I've been told the Leica store in my city is purchasing the gear they have for sale. There's no speculation by Leica regards sales, or consignment. So that store would have to take one out of their own stock and make it a display unit.
Very unlikely to happen.Months from now another store will have a Q2 on display, but Leica will have provided that demo unit and paid Leica for the display space. Mais78:Frankly because Leica releases engender a good deal of false claims about having 'tried it'.That you don't know this about Leica releases just means you don't pay attention to Leica.Above I've avoided the word 'lie' and 'liar'. I simply think most are just wrong. The Q is basically visually indistinguishable from the Q2.Then for example the link by screenname apestorm says nothing about availability of a display unit. But clicking further makes very clear that the camera is NOT IN STOCK.I can provide multiple links for stores in my city that will stock, sell, and display, this camera one once it ships. In some instances such links will note if the camera is on display in the store.Except for perhaps a Leica Store in Berlin, owned by Leica, I doubt very much that any store selling the Q2 will waste sales on a display unit. I made this very clear in my first post.
All Q2s that have shipped are sold immediately.Some times, a Leica rep will have one in a store. Apestorm:'Far more civilised to have a demo unit, get people into the shop to compare it to their current camera, cup of coffee, talk about trade-ins, finance packages, accessories, bags, etc.' I agree, but that simply doesn't seem to be how Leica works its sales in the USA. It takes months for stores selling Leica gear to get demo units.I think the most briskist trade for this camera will come from those who have money and already own a Q that they like a great deal. They will have pre-ordered this camera for shipment to their regular gear store or home or business as soon as the Q2 released.For example screen name marcellodelzio above.However, even if I had the dollars, I'd wait for firmware 1.1.0. Mais78:No, the USA is not the world, but I sure see Leicas on the streets in my US city, and several retailers in my city sell them new and display them.' You should trust people more and not imply their are lieing'We've gone over this before, and in fact that you claim I implied 'they were lying' is now a lie of yours, since I made it very clear what I supposed had occurred, that they mistaken the Q for the Q2.Several other commenters in this thread also didn't believe that anyone had handled a Q2 in a shop, since it really hasn't shipped.You should try reading a bit more before commenting.
Snoddas:All decent lenses are sharp. It's of course assumed the best lenses are sharp in the centre, and sharp with the lens all the way open.No one disputes that Nikon can make a sharp lens.Here you go, I don't care if you don't like the 28mm field of view, that wasn't part of your comment:There doesn't appear to be a Milvus 28mm.
So that's the price of the Q2 for YOUR LENS for the Nikon Z7.So your total for body, adapter, and lens: $8500. Plus a B&W UV filter, extra battery, XQD card, card reader, so that's another $450 likely.Then kinda of bigger than the Q or Q2. Snoddas:So you don't have a clue about how much XQD cards and card readers cost.
They ain't just $20.You just didn't take my hint above about sharpness. It's astounding ignorance that you pretend sharpness is the only important quality of a good lens.You are broadly lens ignorant. No amount of whining about the price of the Leica Q2 will change that.Laughably you also be ignorant of the fact that the Nikon Z7 has exactly 1 card slot.
Apparently you confused it with the D850.' Leica is more about religion than common sense.' Want better colour, you buy Leica's and Zeiss's best lenses. Now, both Leica and Zeiss have some colour knowledge which isn't common. But that knowledge makes sense, whether or not you care to understand it.Enjoy your $5200 Otus 28mm lens. It's extraordinary.
I've tried it. It's better than the 55mm and 85mm. My first post was ironic, did you not get that? I know the Z7 has only one card slot, and I would not buy one for that reason, BUT at least it can provide more than one focal length and functional ergonomics.
AND AGAIN you don't need a card reader, the Z7 is supplied with a fully functional USB-cable. I don't want or need 'better' color, I CALIBRATE my cameras and work flow for ACCURATE color like professionals do. Leica colors, Zeiss colors etc. Are only a part of the myth these companies are trying to market and don't have any relevance to professional photography. And BTW, obviously you don't understand the point with having a short flange distance and larger bayonet mount on the Z-cameras, since you are whining about the Otus lenses.
They are not relevant on the Z-mount. Snoddas:'it was irony' is another Leica basher excuse.' BUT at least it can provide more than one focal length and functional ergonomics'The Leica Q has very good ergonomics, you keep making my point about the ignorance of Leica bashers.'
AND AGAIN you don't need a card reader, the Z7 is supplied with a fully functional USB-cable. I don't want or need 'better' color, I CALIBRATE my cameras and work flow for ACCURATE color like professionals do.' Not if the camera is doing something else. Also you clearly don't know much about colour if you think I'm referencing a calibrated monitor thing.
I'm not surprised by your ignorance; it's quite common.' And BTW, obviously you don't understand the point with having a short flange distance and larger bayonet mount on the Z-cameras, since you are whining about the Otus lenses.' The Q isn't an ILC body.' Leica colors, Zeiss colors etc. Are only a part of the myth.'
Further colour ignorance. Snoddas:'And it has only one cards slot BTW.' Name a 2016/17/18/19 camera with a lens permanently affixed that has two card slots? (With some work, you may be able to find an example from back in 2003/4.)'Looks before ergonomics, not my cup of tea.' No, the Leica Q has both very good ergonomics and a slick look. Yes, I understand that you don't care about either.
Because if you did you'd acknowledge the fact that the Q has both.The Nikon Z7/6, Leica M, S, and SL all have very good ergonomics. So too the Leica CL.
Even the TL2 also. Wolfloid:That's a good NIkon lens. Do you have a link?Lens testing can be very helpful, unlike DXO sensor scoring, but there are things that lens tests don't measure yet, and you can see these things.That good Nikon lens has the standard yellow trouble of good Nikon lenses.Whereas the Qs lens doesn't have any significant colour cast problems. Nor really do good Zeiss lenses, well some sort of do, but it's not as significant as Nikon's issue. Canon's problem is a cyan hue.No, one can't just remove these colour problems without altering the colour-light of the whole image.With SigmaARTs it's blue. With lower level Leicas it's red or magenta.
With Fuji the trouble is blue-green. Snoddas:I've tried all the lenses I reference. I'm not sharing my raws.You managed to source the unbelievable monitor calibration ignorance, by making that claim yourself.Look, you repeated, and added something about changing how a camera (which often isn't a lens) captures jpegs.' And apparently you don't know the difference in camera and monitor profiling.' So digital camera, lens, and colour ignorance combined from you.'
Debating with trolls is not fruitful so I have to say good luck with your trolling.' There's no debate, you lost when you went with 'but monitor calibration'. You showed yourself as caring little about colour and lenses. Canon, Nikon, Sigma-all make fine lenses for your ilk. So at its 'lower priced' end does Leica for the M bodies.
Snoddas:Here's a tip since you're not real up on lenses and colour:Lens tests are helpful, but they largely don't measure what I'm referencing. It ain't transmission curves.Look, your 'but monitor calibration' thing gave you away as not real informed about lenses and colour.You're making a big mistake by thinking this is simply a digital raw extraction thing.
This is further evidence that you know little of lenses and colour.Nor apparently did you read my list above, I'll repost it:Whereas the Qs lens doesn't have any significant colour cast problems. Nor really do good Zeiss lenses, well some sort of do, but it's not as significant as Nikon's issue. Canon's problem is a cyan hue.No, one can't just remove these colour problems without altering the colour-light of the whole image.With SigmaARTs it's blue.
With lower level Leicas it's red or magenta. With Fuji the trouble is blue-green.Final hint: Canon L lenses oft have good colour stopped down massively. Snoddas:My sources are having tried all of the lens lines I mention above. Also owned some of them.Another separate source is your ignorance about 'monitor calibration'; that in and of itself proves you know little about colour.You'll have to figure out why transmission curves only take you so far in judging higher end lenses. I will not explain the reason to you. But the Canon L lens point should give you a hint.Enjoy wondering why so many good photographers' final images look so much better. The lens (if you were buying it) is far more expensive than the Q2.
Even better value if you buy the Q or Q-P. Leicas depreciate at a slower rate than other brands, and they can be serviced for many years.However, if you have invested a lot of money on a particular brand, it is sometimes better to stay with that brand. Professional photographers (those who take photographs for a living) have other priorities in terms of return on investment, and so forth.Finally, R&D is very expensive and camera companies need their devotees who will buy these special editions to get investment for future cameras and lenses. All segments of the market are needed to keep the show on the road - it is shortsighted for one group of users to denigrate another.
Great to have choices and that camera companies manage to stay afloat against the mobile camera market.In the end, the most iconic pictures were taken with very basic cameras by today’s standards. Leica and 'hugely popular'. Ain't that an ixymoron?Winder how many units of the Q v1 they managed to sell globally. 1000 or 10.000? Dont think it was more. Universe of well-heeled Leica collectors is limited, err 'exclusive'. Definitely not 'popular'.
Lol.personally i find a digital camera with a bolted-on fixed lens a 'perversion' when it would be so much more universally capable with a lens mlunt. And almost 'sinful' in terms of sustainability.
In only 3 short years sensor will be outdated and the expensive lens will be retired along with the entire camera. I try to imagine who wants a 47mp sensor camera with a fixed wide angle lens. I doubt that a pro wants it. So that leaves amateurs who will snap candids of friends and family and possibly some 'street photography'.
Then they'll post the pics on social media and email them.But pics viewed on screens look the same whether they were shot on this 47mp full frame sensor, or a smartphone. And they already own the smartphone which is easier to pocket.
So after a 'honeymoon' the Leica will stay at home, and the smartphone will take over. DeepiR:True, I don't have to believe, since I have raws from both the RX1RII and the Q.An additional reason I don't have to believe your claims: Your massive error about the non-existence of the Sony RX1.' You don't own any of these cameras, else you would have posted a few samples on Leica and Sony forums by now.'
Another thing that tells me you know nothing of either, or anything about my habits regards posting.' Actually you haven't even used any of them. And that is a fact.' It clearly makes you insecure that I've tried all 4 Sonys and the Q. Yes, there are 4 Sonys, but the most important thing here is that all 4 have the not great for colour lens.' Irrelevant, they are selling RX1RII now.'
If that were a relevant point, and it's not, then any comment you make about the Q would be irrelevant. Instead, your Q comments are relevant to your Q and Sony ignorance.
I try to imagine who wants a 47mp sensor camera with a fixed wide angle lens. I doubt that a pro wants it. So that leaves amateurs who will snap candids of friends and family and possibly some 'street photography'.
Then they'll post the pics on social media and email them.But pics viewed on screens look the same whether they were shot on this 47mp full frame sensor, or a smartphone. And they already own the smartphone and it's easier to pocket. So after a 'honeymoon' the Leica will stay at home, and the smartphone will take over. I appreciate that Leica has come up with a clean looking revision of the already clean looking Q. But, IMO, elimination of dials and buttons, relegating functions to a touch screen menu can really go too far.
They did the same thing to the TL2 which I bought by trading in my T. Frankly I miss the convenience of switching from single frame to continuous frame by simply flipping a simple switch. Happily, the TL2 retained the buttons on the back for accessing frequently used other functions. Now on the Q2, I fear they have gone a step too far by deleting some important simple buttons on the back.
Nevertheless, the technical features such as improved firmware, higher resolution, additional framing options, etc. Make this a very attractive reportage and travel camera. RK:The more megapixels thing gave you away as highly 'educated'.Now I think you needs learn some things about M lenses and Leica's digital bodies for them.
Those wider M lens capable bodies need a bit more engineering and manufacturing to do the job.Fun fact about the Leica M10, one you seem unfamiliar with: This best digital M yet came out pretty much exactly 2 years ago. Leica is unlikely to rush out a better-err higher pixel-count version, just because pixel counters like you say so.Canon, Sony, and Nikon all sell full framed bodies with fewer than 20MP new in 2019. And yes, the Nikon Df remains the 2nd or 3rd best higher ISO body one can buy-besting both an A9+A7SII.Then Leica, Sony, and Nikon all ship excellent full framed 24MP bodies.Beyond about 10MP, pixel count really doesn't matter unless you're shooting for huge posters, or know that your going to zoom by cropping. And in both of those cases, it's really the lens quality that matters more than pixel count.
RK:This is you above calling the Nikon D5 and Canon 1DXII 'beginners cameras'.' OMG, you are the one who is mired in 2003. Yes, all of these companies have cameras with lower MPs for beginners or people on budgetary restraint which you come across as both.'
See, your problem is you used the plural 'these', which has to reference Sony, Nikon, and Canon and the bodies I listed.' The D5 & 1DxII have lower MPs so that the files can be recorded and moved to storage at 14+ fps, a higher priority for users of those cameras than ultimate IQ.' I see you don't know much about higher IQ. It's basically never a function of more than about 10MP.Now, the big thing for higher IQ in the real world is higher ISO performance, which having less pixel density often increases. It's a pro shooter thing you clearly don't know much about.I see here you omitted the two very not beginner cameras, the Sony A7SII and the Nikon Df, both of which I listed above.Oh, to increase FPS, lower bit depth.
@whitebeardIt looks expensive because A; it's a lot of cheeseburgers, B; you have an interest in photography. Think of all the people that buy cameras that don't have a passion for it, and usually pickup something that does the job like a rebel. Now think of all the people who in the world that own yachts, Ferraris, fly first class, take the helicopter to avoid peak hour, that aren't passionate about photography but still buy a camera. The target market extends beyond photographers for a lot of Leica's sales.
Besides, $5k isn't out of reach for most of middle class, even if it's a lot for what it is. It's really no different than the red flash on the grip of a Nikon, or the red ring around Canon L glass.Nothing to do with German social attitudes, just a brand recognition feature.
Whether you associate any particular brand recognition feature or logo with quality, cost, or anything else, is a factor of that brands reputation.In the case of Leica, their reputation just happens to be one of very high build quality, individualistic styling and high quality optics (plus several negative aspects, of course). Leica 'high-quality optics' doesn't include past sensors (or present optics, according to the astute commentator below). Just compare any Leica with any other good Japanese camera using the DPR studio scene comparison tool and you will consistently find that Leica is NEVER worth the $5,000 or $10,000 they insanely demand for their 'high status' (and admittedly good-looking) camera bodies. German Leica digital cameras are overrated photographically and are not truly worth the price demanded if pictoriographic excellence is your goal. On the one hand I don't think it's that crazily priced considering the normal price of Leicas or (as others have pointed out) even the price of a FF camera + lens these days.OTOH, GFX 50R with the 45mm is almost the same price.
And from the samples I've seen, I definitely prefer the IQ out of the GFX. Different types of cameras of course. I personally shelled out for a Q1, but if I was making that decision today I'd be really tempted to try out the GFX instead and maybe just add a Ricoh GR for compact shooting.